Discussion:
Of note in this case report is the fact that crizanlizumab, originally
approved by the FDA for the treatment of sickle cell-associated
vaso-occlusive crisis based on the SUSTAIN trial[6], appears to have
played an important role in the complete disappearance of priapism in
our patient. This finding, although an isolated case, provides a new
direction for the study and treatment of priapism in SCD patients and
warrants further investigation using real-world data.
The infusion-related reaction (IRR) that occurred in our patient during
the second crizanlizumab infusion, while concerning, is consistent with
previous reports of rare but still significant adverse events during the
initial phase of this treatment regimen. In our patient’s experience,
the occurrence of IRR is not indicative of a recurrence of the episode
and does not compromise the long-term benefit of treatment. This finding
should encourage physicians to consider continuing crizanlizumab
treatment even after a single IRR event, no doubt after careful
risk-benefit assessment [7]. However, our case emphasises the urgent
need for guidance on the management of such infusion-related reactions.
The overall results of crizanlizumab administration in our patient were
positive, confirming its benefit in the management of vaso-occlusive
crises in SCD and its potential benefit in stuttering priapism.
Nevertheless, future studies with a larger cohort and longer follow-up
periods are urgently needed to validate these results and to better
understand the unseen facets of crizanlizumab therapy.